Abstract：
Chordomas are a rare and relatively slow-growing malignancy of notochordal origin with a nearly 50% recurrence rate. Chordomas of the cervical spine are particularly challenging tumors given surrounding vital anatomical structures. Although standard in other areas of the spine, en bloc resection of cervical chordomas is exceedingly difficult and carries the risk of significant postoperative morbidity. Here, the authors present their institutional experience with 13 patients treated with a structure-sparing radical resection and adjuvant radiation for cervical chordomas.
Records of the standing senior author and institutional database of spinal surgeries were retrospectively reviewed for surgically managed cervical and high thoracic chordomas between 1997 and 2022. Chordomas whose epicenter was cervical but touched the clivus or had extension to the thoracic spine were included in this series. Clinical and operative data were gathered and analyzed for the index surgery and any revisions needed. Outcome metrics such as recurrence rates, complication rates, functional status, progression-free interval (PFI) and overall survival (OS) were evaluated.
The median patient age at diagnosis was 57 (range 32-80) years. The median modified Rankin Scale (mRS) score at the time of presentation was 1 (range 0-4). Approximately 40% of tumors were located in the upper cervical spine (occiput-C2). The median time from diagnosis to surgery was 74.5 (range 10-483) days. Gross-total resection was achieved in just under 40% of patients. All patients received adjuvant radiotherapy. The mean duration of follow-up was 4.09 years, with a mean PFI of 3.80 (range 1.16-13.1) years. Five patients experienced recurrence (38.5%). The mean OS was 3.44 years. Three patients died during the follow-up period; 2 due to disease progression and 1 died in the immediate postoperative period. One patient was lost to follow-up. A significant positive relationship was identified between high cervical tumor location and disease recurrence (p = 0.021).
While en bloc resection is appropriate and feasible for tumors in the sacral spine, the cervical region poses a significant technical challenge and is associated with increased postoperative morbidity. Radical resection may allow for achievement of negative operative margins and, along with sparing postoperative morbidity following resection of cervical chordomas, maintaining a similar rate of recurrence when compared with en bloc resection while preserving quality of life.
Records of the standing senior author and institutional database of spinal surgeries were retrospectively reviewed for surgically managed cervical and high thoracic chordomas between 1997 and 2022. Chordomas whose epicenter was cervical but touched the clivus or had extension to the thoracic spine were included in this series. Clinical and operative data were gathered and analyzed for the index surgery and any revisions needed. Outcome metrics such as recurrence rates, complication rates, functional status, progression-free interval (PFI) and overall survival (OS) were evaluated.
The median patient age at diagnosis was 57 (range 32-80) years. The median modified Rankin Scale (mRS) score at the time of presentation was 1 (range 0-4). Approximately 40% of tumors were located in the upper cervical spine (occiput-C2). The median time from diagnosis to surgery was 74.5 (range 10-483) days. Gross-total resection was achieved in just under 40% of patients. All patients received adjuvant radiotherapy. The mean duration of follow-up was 4.09 years, with a mean PFI of 3.80 (range 1.16-13.1) years. Five patients experienced recurrence (38.5%). The mean OS was 3.44 years. Three patients died during the follow-up period; 2 due to disease progression and 1 died in the immediate postoperative period. One patient was lost to follow-up. A significant positive relationship was identified between high cervical tumor location and disease recurrence (p = 0.021).
While en bloc resection is appropriate and feasible for tumors in the sacral spine, the cervical region poses a significant technical challenge and is associated with increased postoperative morbidity. Radical resection may allow for achievement of negative operative margins and, along with sparing postoperative morbidity following resection of cervical chordomas, maintaining a similar rate of recurrence when compared with en bloc resection while preserving quality of life.
摘要：
目的：脊索瘤是一种罕见且生长相对缓慢的脊索起源恶性肿瘤，复发率接近50%。考虑到周围重要的解剖结构，颈椎的脊索瘤是特别具有挑战性的肿瘤。虽然在脊柱的其他区域是标准的，宫颈脊索瘤的整块切除术非常困难，并且存在严重的术后发病率的风险。这里,作者介绍了13例保留结构的根治性切除和辅助放疗治疗宫颈脊索瘤的机构经验.
方法：回顾性分析了1997年至2022年期间手术治疗的颈部和高胸椎脊索瘤的脊柱手术记录和机构数据库。该系列包括震中为宫颈但触及斜坡或延伸至胸椎的脊索瘤。收集并分析了临床和手术数据，以进行索引手术和所需的任何修订。结果指标，如复发率，并发症发生率,功能状态，评估无进展间期(PFI)和总生存期(OS).
结果：诊断时患者年龄中位数为57岁（范围32-80岁）。演示时的中位改良Rankin量表（mRS）评分为1（范围0-4）。大约40％的肿瘤位于上颈椎（枕骨C2）。从诊断到手术的中位时间为74.5天（范围10-483天）。不到40％的患者实现了总切除。所有患者均接受辅助放疗。平均随访时间为4.09年,平均PFI为3.80(范围为1.16-13.1)年。5例患者复发（38.5%）。平均OS为3.44年。3例患者在随访期间死亡；2例因疾病进展而死亡，1例在术后即刻死亡。一名患者失去了随访。宫颈高位肿瘤位置与疾病复发之间存在显着正相关（p=0.021）。
结论：虽然整块切除对于骶骨脊柱肿瘤是合适且可行的，宫颈区域构成了重大的技术挑战，并且与术后发病率增加有关。根治性切除可以实现阴性手术切缘，同时保留宫颈脊索瘤切除术后的术后发病率，与整块切除术相比，保持相似的复发率，同时保持生活质量。
方法：回顾性分析了1997年至2022年期间手术治疗的颈部和高胸椎脊索瘤的脊柱手术记录和机构数据库。该系列包括震中为宫颈但触及斜坡或延伸至胸椎的脊索瘤。收集并分析了临床和手术数据，以进行索引手术和所需的任何修订。结果指标，如复发率，并发症发生率,功能状态，评估无进展间期(PFI)和总生存期(OS).
结果：诊断时患者年龄中位数为57岁（范围32-80岁）。演示时的中位改良Rankin量表（mRS）评分为1（范围0-4）。大约40％的肿瘤位于上颈椎（枕骨C2）。从诊断到手术的中位时间为74.5天（范围10-483天）。不到40％的患者实现了总切除。所有患者均接受辅助放疗。平均随访时间为4.09年,平均PFI为3.80(范围为1.16-13.1)年。5例患者复发（38.5%）。平均OS为3.44年。3例患者在随访期间死亡；2例因疾病进展而死亡，1例在术后即刻死亡。一名患者失去了随访。宫颈高位肿瘤位置与疾病复发之间存在显着正相关（p=0.021）。
结论：虽然整块切除对于骶骨脊柱肿瘤是合适且可行的，宫颈区域构成了重大的技术挑战，并且与术后发病率增加有关。根治性切除可以实现阴性手术切缘，同时保留宫颈脊索瘤切除术后的术后发病率，与整块切除术相比，保持相似的复发率，同时保持生活质量。
